{"id":4566,"date":"2022-08-10T22:23:19","date_gmt":"2022-08-10T14:23:19","guid":{"rendered":"http:\/\/dh.oncolab.cn\/index.php\/2022\/08\/10\/%e5%85%8d%e7%96%ab%e5%ad%a6%e6%8b%be%e9%81%97-%e7%ac%ac32%e7%ab%a0%ef%bc%9a%e5%8e%9f%e5%8f%91%e6%80%a7%e5%85%8d%e7%96%ab%e7%bc%ba%e9%99%b7%e7%97%85\/"},"modified":"2022-08-10T22:23:19","modified_gmt":"2022-08-10T14:23:19","slug":"%e5%85%8d%e7%96%ab%e5%ad%a6%e6%8b%be%e9%81%97-%e7%ac%ac32%e7%ab%a0%ef%bc%9a%e5%8e%9f%e5%8f%91%e6%80%a7%e5%85%8d%e7%96%ab%e7%bc%ba%e9%99%b7%e7%97%85","status":"publish","type":"post","link":"http:\/\/dh.oncolab.cn\/index.php\/2022\/08\/10\/%e5%85%8d%e7%96%ab%e5%ad%a6%e6%8b%be%e9%81%97-%e7%ac%ac32%e7%ab%a0%ef%bc%9a%e5%8e%9f%e5%8f%91%e6%80%a7%e5%85%8d%e7%96%ab%e7%bc%ba%e9%99%b7%e7%97%85\/","title":{"rendered":"\u514d\u75ab\u5b66\u62fe\u9057-\u7b2c32\u7ae0\uff1a\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5"},"content":{"rendered":"
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\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5<\/strong>\u662f\u6307\u514d\u75ab\u7cfb\u7edf\u51fa\u73b0\u90e8\u5206\u7f3a\u5931\u6216\u529f\u80fd\u5f02\u5e38<\/strong>\u7684\u75be\u75c5\u3002\u5728\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u4e2d\uff0c\u7f3a\u9677\u662f\u7531\u514d\u75ab\u7cfb\u7edf\u672c\u8eab\u5f15\u8d77\uff0c\u800c\u4e0d\u662f\u5176\u4ed6\u75be\u75c5\u3001\u6bd2\u7d20\u6216\u836f\u7269\u5f15\u8d77\u7684\u7ee7\u53d1\u6027\u75be\u75c5\uff0c\u7ee7\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u5c06\u5728\u7b2c33\u7ae0\u4e2d<\/span>\u8be6\u7ec6\u4ecb\u7ecd<\/span><\/span>\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u76ee\u524d\u5df2\u53d1\u73b0100\u591a\u79cd\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\uff0c\u5176\u4e2d\u5927\u90e8\u5206\u662f\u7531\u7a81\u53d8<\/strong>\u5f15\u8d77\u7684\u3002\u5728\u524d\u9762\u7684\u7ae0\u8282\u4e2d\u5df2\u7ecf\u4ecb\u7ecd\u4e86\u5176\u4e2d\u7684\u4e00\u4e9b\u75be\u75c5\uff0c\u672c\u7ae0\u4e5f\u4f1a\u5bf9\u6b64\u8fdb\u884c\u56de\u987e\u3002\u6b64\u5916\uff0c\u5c11\u6570\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u662f\u7531\u81ea\u8eab\u514d\u75ab<\/strong>\u5f15\u8d77\u7684\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u53ef\u80fd\u5bfc\u81f4\u673a\u4f53\u6613\u53d7\u5230\u611f\u67d3\u548c\u80bf\u7624\u4fb5\u88ad<\/strong>\u3002\u672c\u7ae0\u4ecb\u7ecd\u4e86\u4e0d\u540c\u7c7b\u578b\u7684\u611f\u67d3\u6240\u4ee3\u8868\u7684\u4e0d\u540c\u7c7b\u578b\u514d\u75ab\u7f3a\u9677\u53ca\u5176\u4e25\u91cd\u7a0b\u5ea6\uff0c\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u7684\u8bca\u65ad\u548c\u6cbb\u7597\u4e5f\u4f1a\u6709\u6240\u6d89\u53ca\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u611f\u67d3\u7c7b\u578b\u6709\u52a9\u4e8e\u5224\u65ad\u514d\u75ab\u7f3a\u9677\u7c7b\u578b<\/strong><\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u5728\u5e7c\u513f\u65f6\u671f\uff0c\u673a\u4f53\u5bf9\u5e38\u89c1\u75c5\u539f\u4f53\u7684\u514d\u75ab\u529b\u5c1a\u4e0d\u5b8c\u5584\u3002\u56e0\u6b64\uff0c\u5e7c\u513f\u5728\u51ac\u5929\u6709\u4e09\u5230\u56db\u6b21\u4e0a\u547c\u5438\u9053\u611f\u67d3\u662f\u5f88\u6b63\u5e38\u7684\u3002\u4f46\u5e7c\u513f\u82e5\u51fa\u73b0\u8fde\u7eed\u3001\u53cd\u590d\u53d1\u4f5c\u7684\u4e0a\u547c\u5438\u9053\u611f\u67d3\u6216\u9635\u53d1\u6027\u80ba\u708e\uff0c\u5219\u4e0d\u5e38\u89c1\u3002\u5065\u5eb7\u6210\u5e74\u4eba\u4e5f\u4f1a\u65f6\u4e0d\u65f6\u5730\u53d7\u5230\u611f\u67d3\uff0c\u572820\u5c81\u523060\u5c81\u671f\u95f4\uff0c\u5927\u591a\u6570\u5065\u5eb7\u7684\u4eba\u6bcf\u5e74\u90fd\u4f1a\u7ecf\u5386\u4e24\u5230\u4e09\u6b21\u611f\u67d3\u3002\u5176\u4e2d\u5927\u591a\u6570\u5728\u6ca1\u6709\u4efb\u4f55\u533b\u7597\u5e72\u9884\u7684\u60c5\u51b5\u4e0b\u75c5\u60c5\u4f1a\u81ea\u7136\u597d\u8f6c\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u7136\u800c\uff0c\u53cd\u590d\u3001\u7f55\u89c1\u6216\u96be\u4ee5\u6cbb\u7597\u7684\u611f\u67d3\u662f\u514d\u75ab\u7f3a\u9677\u7684\u4e00\u4e2a\u91cd\u8981\u6807\u5fd7<\/strong>\uff0c\u4efb\u4f55\u9700\u8981\u591a\u7597\u7a0b\u6297\u751f\u7d20\u6cbb\u7597\u6216\u56e0\u611f\u67d3\u4f4f\u9662\u7684\u4eba\u90fd\u5e94\u6392\u67e5\u8fd9\u4e00\u53ef\u80fd\u6027\u3002\u60a3\u8005\u6240\u53d7\u5230\u7684\u611f\u67d3\u7c7b\u578b\u53ef\u4ee5\u4e3a\u9020\u6210\u514d\u75ab\u7f3a\u9677\u7684\u539f\u56e0\u63d0\u4f9b\u7ebf\u7d22\uff08Fig.32.1<\/span>\uff09\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u53cd\u590d\u611f\u67d3\u5305\u819c\u7ec6\u83cc\u662f\u6297\u4f53\u751f\u6210\u7f3a\u9677<\/strong>\u7684\u6807\u5fd7\uff0c\u56e0\u4e3a\u6297\u4f53\u662f\u6d88\u706d\u80de\u5916\u7ec6\u83cc\u7684\u5173\u952e\u56e0\u7d20\u3002\u6297\u4f53\u3001\u514d\u75ab\u7403\u86cb\u767dG\uff08IgG\uff09\u548cIgA\u5728\u62b5\u6297\u547c\u5438\u9053\u611f\u67d3\u4e2d\u8d77\u4e3b\u8981\u9632\u5fa1\u4f5c\u7528\uff0c\u800c\u6297\u4f53\u7f3a\u4e4f\u4f1a\u5bfc\u81f4\u7531\u80ba\u708e\u7403\u83cc\u6216\u55dc\u80ba\u83cc\u5f15\u8d77\u7684\u53cd\u590d\u6027\u547c\u5438\u9053\u611f\u67d3\uff0c\u4ece\u800c\u5bfc\u81f4\u652f\u6c14\u7ba1\u4e0d\u53ef\u9006\u635f\u5bb3\u6216\u652f\u6c14\u7ba1\u6269\u5f20\u3002<\/p>\n

\u8461\u8404\u7403\u83cc\u3001\u9769\u5170\u6c0f\u9634\u6027\u83cc\u548c\u771f\u83cc\u611f\u67d3\u4f1a\u4f7f\u541e\u566c\u7ec6\u80de\u6570\u91cf\u51cf\u5c11\u6216\u529f\u80fd\u5f02\u5e38\u3002\u7531\u4e8e\u67d0\u4e9b\u672a\u77e5\u539f\u56e0\uff0c\u67d0\u4e9b\u8865\u4f53\u7f3a\u9677\u6613\u5bfc\u81f4\u8111\u819c\u708e\u5948\u745f\u83cc\u5f15\u8d77\u7684\u8111\u819c\u708e\uff08\u89c1\u7b2c20\u7ae0<\/span>\uff09\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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T\u7ec6\u80de\u548c\u5de8\u566c\u7ec6\u80de\u5728\u8bc6\u522b\u548c\u6d88\u9664\u7ec6\u80de\u5185\u611f\u67d3\u65b9\u9762\u5177\u6709\u7279\u6b8a\u4f5c\u7528\u3002T\u7ec6\u80de\u6216\u5de8\u566c\u7ec6\u80de\u7f3a\u9677\u6613\u5bfc\u81f4\u673a\u4f53\u611f\u67d3\u7ec6\u80de\u5185\u75c5\u539f\u4f53<\/strong>\uff0c\u5982\u539f\u751f\u52a8\u7269\u3001\u75c5\u6bd2\u548c\u7ec6\u80de\u5185\u7ec6\u83cc\uff0c\u5305\u62ec\u5206\u679d\u6746\u83cc\uff08\u89c1Fig.32.1<\/span>\uff09\u3002<\/p>\n

\u6f5c\u4f0f\u6027\u75b1\u75b9\u75c5\u6bd2\u7684\u518d\u6fc0\u6d3b\u4e0eT\u7ec6\u80de\u514d\u75ab\u7f3a\u9677\u5c24\u4e3a\u76f8\u5173\uff1b\u53cd\u590d\u53d1\u4f5c\u7684\u51bb\u75ae\uff08\u5355\u7eaf\u75b1\u75b9\uff09\u6216\u5e26\u72b6\u75b1\u75b9\uff08\u6c34\u75d8\u5e26\u72b6\u75b1\u75b9\uff09\u8868\u660e\u673a\u4f53\u53ef\u80fd\u5b58\u5728\u8f7b\u5ea6\u514d\u75ab\u7f3a\u9677\uff1b\u590d\u53d1\u6027\u5ff5\u73e0\u83cc\u611f\u67d3\u4ee3\u8868TH17\u901a\u8def\u5b58\u5728\u7f3a\u9677\uff1b\u75b1\u75b9\u75c5\u6bd2\u8bf1\u5bfc\u7684\u80bf\u7624\uff0c\u5c24\u5176\u662f\u5361\u6ce2\u897f\u8089\u7624\uff08\u4eba\u7c7b\u75b1\u75b9\u75c5\u6bd28\u578b[HHV8]\uff09\u548c\u975e\u970d\u5947\u91d1\u6dcb\u5df4\u7624\uff08EB\u75c5\u6bd2[EBV]\uff09\u662fT\u7ec6\u80de\u529f\u80fd\u969c\u788d\u7684\u7279\u5f81\u3002\u7b2c35\u7ae0<\/span>\u5c06\u8fdb\u4e00\u6b65\u8ba8\u8bba\u8fd9\u4e9b\u95ee\u9898\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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T\u7ec6\u80de\u514d\u75ab\u7f3a\u9677\u7684\u4e25\u91cd\u7a0b\u5ea6\u4e5f\u53cd\u6620\u5728\u5206\u679d\u6746\u83cc\u611f\u67d3\u7684\u65b9\u5f0f\u4e2d<\/strong>\uff08\u89c1Fig.32.1<\/span>\uff09\u3002\u7ed3\u6838\u5206\u679d\u6746\u83cc\uff08mycobacterium tuberculosis\uff09\u662f\u4e00\u79cd\u6bd2\u529b\u5f88\u5f3a\u7684\u75c5\u539f\u4f53\uff0c\u53ef\u5bfc\u81f4\u514d\u75ab\u529f\u80fd\u6b63\u5e38\u7684\u4eba\u53d1\u751f\u80ba\u90e8\u611f\u67d3\u3002\u800c\u5728\u8f7b\u5ea6T\u7ec6\u80de\u514d\u75ab\u7f3a\u9677\u4e2d\uff0c\u8be5\u75c5\u539f\u4f53\u66f4\u662f\u80fd\u591f\u5165\u4fb5\u9664\u80ba\u90e8\u4ee5\u5916\u7684\u5176\u4ed6\u90e8\u5206\u3002\u66f4\u4e25\u91cd\u7684\u514d\u75ab\u7f3a\u9677\u5219\u4f1a\u5bfc\u81f4\u5728\u73af\u5883\u4e2d\u5e38\u89c1\u7684\u4f4e\u6bd2\u5206\u679d\u6746\u83cc\uff08\u4f8b\u5982\u9e1f\u5206\u679d\u6746\u83cc\u80de\u5185\u590d\u5408\u4f53\uff09\u7684\u611f\u67d3\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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Fig.32.1 <\/span>\u673a\u4f1a\u6027\u611f\u67d3\u7684\u7c7b\u578b\u4e5f\u53ef\u4ee5\u5e2e\u52a9\u5224\u65ad\u9020\u6210\u514d\u75ab\u7f3a\u9677\u7684\u539f\u56e0\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u539f<\/strong>\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u7684\u75c5\u56e0<\/strong><\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u7684\u539f\u56e0\u53ef\u5206\u4e3a\uff1a<\/p>\n

\u2022\u7a81\u53d8<\/strong>\uff1a<\/strong>\u7a81\u53d8\u672c\u8eab\u6982\u7387\u8f83\u4f4e\uff0c\u4f46\u5b83\u53ef\u80fd\u5f71\u54cd\u514d\u75ab\u7cfb\u7edf\u7684\u4efb\u4f55\u90e8\u5206\uff0c\u5e76\u5bfc\u81f4\u4e25\u91cd\u75be\u75c5\u3002<\/p>\n

\u2022\u591a\u6001\u6027\uff1a<\/strong>\u5f71\u54cd\u514d\u75ab\u7cfb\u7edf\u6240\u6709\u90e8\u5206\u5e76\u5bfc\u81f4\u611f\u67d3\u7684\u98ce\u9669\u589e\u52a0\uff0c\u8fd9\u662f\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u7684\u5e38\u89c1\u7279\u5f81\u3002<\/p>\n

\u2022\u591a\u57fa\u56e0\u75be\u75c5\uff1a<\/strong>\u8fd9\u4e9b\u76f8\u5bf9\u5e38\u89c1\u7684\u75be\u75c5\u4e3b\u8981\u5f71\u54cd\u6297\u4f53\u3002\u5176\u4e2d\u4e00\u4e9b\u591a\u57fa\u56e0\u75be\u75c5\u53ef\u80fd\u662f\u7531\u81ea\u8eab\u514d\u75ab\u5f15\u8d77\u7684\u3002<\/p>\n

\u4e0a\u8ff0\u75c5\u56e0\u5728 Table 32.1<\/span>\u548cFig.32.2<\/span>\u4e2d\u8fdb\u884c\u4e86\u603b\u7ed3\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u7a81\u53d8\u4e0e\u514d\u75ab\u7f3a\u9677<\/p>\n<\/section>\n

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\u76ee\u524d\u5df2\u77e5\u6709100\u591a\u79cd\u5bfc\u81f4\u514d\u75ab\u7f3a\u9677\u7684\u7a81\u53d8\u3002\u672c\u4e66\u7740\u91cd\u4ecb\u7ecd\u5bfc\u81f4\u514d\u75ab\u7f3a\u9677\u7684\u5341\u51e0\u79cd\u9002\u5e94\u6027\u514d\u75ab\u7cfb\u7edf\u76f8\u5173\u57fa\u56e0\u7684\u91cd\u8981\u7a81\u53d8\u3002\u8bb8\u591a\u7a81\u53d8\u4f1a\u5bfc\u81f4\u4e25\u91cd\u8054\u5408\u514d\u75ab\u7f3a\u9677\uff08SCID\uff09<\/strong>\uff0c\u8fd9\u662f\u4e00\u79cd\u540c\u65f6\u5f71\u54cdT\u7ec6\u80de\u548cB\u7ec6\u80de\u7684\u75be\u75c5\u3002\u4e0d\u540c\u7c7b\u578b\u7684SCID\u5747\u53ef\u4ee5\u8868\u660e\u673a\u4f53\u5b58\u5728\u975e\u5e38\u4e25\u91cd\u7684\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\uff0c\u5176\u53ef\u7531\u7ec6\u80de\u56e0\u5b50\u03b3\u94fe\u53d7\u4f53\u7f3a\u9677\u548cRAG\u7a81\u53d8<\/strong>\u5f15\u8d77\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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SCID\u5f88\u4e25\u91cd\uff0c\u56e0\u4e3a\u60a3\u8005\u7684T\u7ec6\u80de\u548cB\u7ec6\u80de\u90fd\u4f1a\u53d7\u5230\u5f71\u54cd\u3002\u60a3\u6709SCID\u7684\u5a74\u513f\u82e5\u4e0d\u7ecf\u8fc7\u6cbb\u7597\uff0c\u5728\u51fa\u751f\u540e\u51e0\u4e2a\u6708\u5185\u5c31\u4f1a\u6b7b\u4ea1\u3002\u5176\u4e2d\u4e00\u4e9b\u75be\u75c5\u662f\u81ea\u53d1\u9057\u4f20\u7684\uff08\u5982RAG\u7f3a\u4e4f\u75c7 \uff0c\u89c1Box 7.1<\/span>\uff09\uff0c\u53ef\u80fd\u6709\u5bb6\u65cf\u53f2\u3002\u5176\u4ed6\u7c7b\u578b\u7684SCID\u5305\u62ecX\u8fde\u9501SCID\uff08\u4f8b\u5982\uff0c\u03b3\u94fe\u7f3a\u9677\uff0c\u89c1Box 12.2<\/span>\uff09\u548c\u9ad8IgM\u7efc\u5408\u5f81\uff08\u89c1Box 16.2<\/span>\uff09\uff0c\u8fd9\u7c7b\u60a3\u8005\u7684\u6bcd\u4eb2\u548c\u8205\u8205\u53ef\u80fd\u6709\u65e9\u4ea1\u53f2\u3002\u800cDiGeorge\u7efc\u5408\u5f81\uff08\u89c1Box 15.1<\/span>\uff09\u662f\u753122\u53f7\u67d3\u8272\u4f53\u5927\u90e8\u5206\u6613\u4f4d\u5230\u5176\u4ed6\u67d3\u8272\u4f53\u4e0a\u5f15\u8d77\u7684\uff0c\u8fd9\u662f\u975e\u9057\u4f20\u7684\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n

\u591a\u6001\u6027\u4e0e\u514d\u75ab\u7f3a\u9677<\/p>\n<\/section>\n

\n
\n
\n

\u9057\u4f20\u591a\u6001\u6027\u662f\u6307\u67d0\u4e2a\u57fa\u56e0\u5ea7\u4e0a\u5b58\u5728\u4e24\u4e2a\u6216\u4e24\u4e2a\u4ee5\u4e0a\u7684\u7b49\u4f4d\u57fa\u56e0\uff0c\u4e14\u7b49\u4f4d\u57fa\u56e0\u7684\u9891\u7387\u5927\u4e8e0.01\u7684\u73b0\u8c61\uff0c\u773c\u775b\u989c\u8272\u5c31\u662f\u9057\u4f20\u591a\u6001\u6027\u7684\u4e00\u4e2a\u4f8b\u5b50\u3002\u4eba\u7c7b\u767d\u7ec6\u80de\u6297\u539f(HLA)\u7b49\u4f4d\u57fa\u56e0\u662f\u591a\u6001\u7684<\/strong>\uff0c\u4f1a\u5bf9\u4e00\u4e9b\u5305\u62ec\u4e59\u809d\u548cHIV\u5728\u5185\u7684\u611f\u67d3\u7684\u4e25\u91cd\u7a0b\u5ea6\u4ea7\u751f\u5f71\u54cd(\u89c1Box 3.1<\/span>)\u3002<\/p>\n

\u82e5\u4eba\u7c7b\u767d\u7ec6\u80de\u6297\u539f(HLA)\u7b49\u4f4d\u57fa\u56e0\u63a7\u5236\u673a\u4f53\u4e0d\u80fd\u4e0e\u75c5\u6bd2\u591a\u80bd\u7ed3\u5408\uff0c\u90a3\u4e48\u8fd9\u7c7b\u4e2a\u4f53\u53d7\u5230\u7684\u611f\u67d3\u4f1a\u66f4\u4e3a\u4e25\u91cd\u3002\u4e0eHIV\u98ce\u9669\u76f8\u5173\u7684\u8d8b\u5316\u56e0\u5b50\u53ca\u5176\u53d7\u4f53\u7684\u591a\u6001\u6027\u5c06\u5728\u7b2c33\u7ae0<\/span>\u4e2d\u8ba8\u8bba\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u7518\u9732\u805a\u7cd6\u7ed3\u5408\u51dd\u96c6\u7d20(MBL)<\/strong>\u662f\u4e00\u79cd\u80f6\u539f\u6837\u86cb\u767d\uff0c\u80fd\u7ed3\u5408\u7ec6\u83cc\u7ec6\u80de\u58c1\u4e2d\u7684\u7cd6\u5e76\u6fc0\u6d3b\u7ecf\u5178\u7684\u8865\u4f53\u9014\u5f84(\u7b2c20\u7ae0<\/span>)\u3002MBL\u548c\u8865\u4f53\u7684\u591a\u6001\u6027\u5bf9\u4e8e\u673a\u4f53\u7684\u611f\u67d3\u4e5f\u4f1a\u4ea7\u751f\u5f71\u54cd(Fig.32.3<\/span>)\u3002\u591a\u6001\u6027\u5bf9\u4e2a\u4f53\u7684\u5f71\u54cd\u5f88\u5c0f\uff0c\u53ea\u6709\u5728\u5bf9\u7fa4\u4f53\u7684\u7814\u7a76\u4e2d\u624d\u8f83\u4e3a\u660e\u663e\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u5728\u53d7\u5230\u4e0d\u540c\u7a0b\u5ea6\u7684\u6d41\u884c\u6027\u611f\u67d3\u5f71\u54cd\u7684\u4eba\u7fa4\u4e2d\uff0c\u591a\u6001\u6027\u4ee5\u4e0d\u540c\u7684\u9891\u7387\u6301\u7eed\u5b58\u5728\u3002\u6700\u8457\u540d\u7684\u4f8b\u5b50\u662f\u8840\u7ea2\u86cb\u767dS\u591a\u6001\u6027<\/strong>(\u9570\u72b6\u7ec6\u80de\u6027\u8d2b\u8840)\u53ef\u9884\u9632\u759f\u75be\uff0c\u5728\u5c45\u4f4f\u6216\u8d77\u6e90\u4e8e\u5e38\u53d1\u759f\u75be\u5730\u533a\u7684\u4eba\u7fa4\u4e2d\u66f4\u4e3a\u5e38\u89c1\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

Table 32.1 <\/span>\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u7684\u539f\u56e0<\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n

\u591a\u57fa\u56e0\u75be\u75c5<\/p>\n<\/section>\n

\n
\n
\n

\u591a\u57fa\u56e0\u75be\u75c5<\/strong>\u662f\u7531\u51e0\u4e2a\u57fa\u56e0\u7684\u76f8\u4e92\u4f5c\u7528\u5f15\u8d77\u7684\uff0c\u73af\u5883\u56e0\u7d20\u4e5f\u8d77\u5230\u4e86\u4e00\u5b9a\u4f5c\u7528\u3002\u5e38\u89c1\u53d8\u5f02\u578b\u514d\u75ab\u7f3a\u9677\u75c5(CVID)\u3001IgA\u7f3a\u9677\u548c\u7279\u5f02\u6027\u6297\u4f53\u7f3a\u9677<\/strong>\u662f\u76f8\u5bf9\u5e38\u89c1\u7684\u591a\u57fa\u56e0\u75be\u75c5\uff0c\u5b83\u4eec\u4e3b\u8981\u5f71\u54cd\u6297\u4f53\u7684\u4ea7\u751f<\/strong>\u3002\u4ec5\u6709\u7ea6\u4e09\u5206\u4e4b\u4e00\u7684\u591a\u57fa\u56e0\u75be\u75c5\u60a3\u8005\u8f83\u6613\u53d7\u5230\u611f\u67d3\uff0c\u5177\u4f53\u539f\u56e0\u8fd8\u4e0d\u6e05\u695a\uff0c\u800c\u5927\u7ea6600\u4eba\u4e2d\u67091\u4eba\u60a3\u6709\u514d\u75ab\u7403\u86cb\u767d\u7f3a\u4e4f\u3002\u5728IgA\u7f3a\u4e4f\u7684\u60a3\u8005\u4e2d\u4e73\u7cdc\u6cfb(\u7b2c28\u7ae0<\/span>)\u66f4\u4e3a\u5e38\u89c1\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

CVID<\/strong>(\u89c1Box 32.1<\/span>)\u7684\u53d1\u75c5\u7387\u7ea6\u4e3a\u6bcf20000\u540d\u5e74\u8f7b\u4eba\u4e2d\u5c31\u67091\u4f8b\uff0c\u5bf9\u7537\u6027\u548c\u5973\u6027\u7684\u5f71\u54cd\u76f8\u5f53\u3002CVID\u662f\u6700\u5e38\u89c1\u7684\u9700\u8981\u5e72\u9884\u7684\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5<\/strong>\uff0c\u60a3\u8005\u7684\u603bIgG\u6c34\u5e73\u8f83\u4f4e\uff0c\u800c\u5176\u4ed6\u7ed3\u679c\uff08\u5982IgA\u548cIgM\u6c34\u5e73\u4ee5\u53caB\u3001T\u7ec6\u80de\u6570\u91cf\uff09\u662f\u53d8\u5316\u7684\u3002<\/p>\n

CVID\u4f1a\u5bfc\u81f4\u53cd\u590d\u53d1\u4f5c\u7684\u547c\u5438\u9053\u611f\u67d3\uff0c\u6d89\u53ca\u80a0\u9053\u3001\u76ae\u80a4\u548c\u795e\u7ecf\u7cfb\u7edf\u7684\u611f\u67d3\u4e5f\u4f1a\u53d1\u751f\u3002\u81ea\u8eab\u514d\u75ab\u5728CVID\u4e2d\u5f88\u5e38\u89c1\uff0c\u901a\u5e38\u5305\u62ec\u6076\u6027\u8d2b\u8840\u548c\u7532\u72b6\u817a\u75be\u75c5\u3001\u5173\u8282\u708e\u548c\u514d\u75ab\u6027\u8840\u5c0f\u677f\u51cf\u5c11\u75c7\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

CVID\u6709\u9057\u4f20\u7684\u53ef\u80fd\uff0c\u8bb8\u591a\u60a3\u8005\u6709\u5bb6\u65cf\u75c5\u53f2\uff0c\u5176\u4e3a\u5e38\u67d3\u8272\u4f53\u9690\u6027\u9057\u4f20\u3002\u7136\u800c\uff0cCVID\u7684\u53d1\u75c5\u8f83\u665a\uff0c\u8fd9\u4ee3\u8868\u6709\u4e0d\u660e\u73af\u5883\u56e0\u7d20\u8d77\u7740\u5173\u952e\u4f5c\u7528\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u5728\u7279\u5f02\u6027\u6297\u4f53\u7f3a\u4e4f\u7684\u60a3\u8005\u4e2d\uff0c\u5c3d\u7ba1\u4ed6\u4eec\u7684\u603bIgG\u6b63\u5e38\uff0c\u4f46\u4ecd\u6709\u53cd\u590d\u611f\u67d3\u80ba\u708e\u7403\u83cc\u6216\u55dc\u8840\u6746\u83cc\u7684\u503e\u5411\uff0c\u5373\u4f7f\u63a5\u79cd\u75ab\u82d7\uff0c\u4ed6\u4eec\u5bf9\u591a\u7cd6\u6297\u539f\u4e5f\u65e0\u53cd\u5e94\uff0c\u5bf9\u80ba\u708e\u7403\u83cc\u6297\u539f\u7684\u6297\u4f53\u6ef4\u5ea6\u4e5f\u5f88\u4f4e\u3002\u7279\u5f02\u6027\u6297\u4f53\u7f3a\u4e4f\u901a\u5e38\u5728\u5a74\u513f\u671f\u548c\u813e\u5207\u9664\u672f\u540e\u77ed\u6682\u51fa\u73b0\uff08\u89c1Box 13.1<\/span>\uff09\uff0c\u4f46\u5b83\u672c\u8eab\u786e\u5b9e\u662f\u4e00\u79cd\u75be\u75c5\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u5176\u4ed6\u4e00\u4e9b\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u662f\u7531\u81ea\u8eab\u514d\u75ab<\/strong>\u5f15\u8d77\u7684\u3002\u4f8b\u5982\uff0c\u81ea\u8eab\u514d\u75ab\u6027\u591a\u5185\u5206\u6ccc\u817a\u75c5-\u5ff5\u73e0\u83cc\u75c5-\u5916\u80da\u5c42\u8425\u517b\u969c\u788d\u75c5\uff08APECED\uff1bBox 15.2<\/span>\uff09\u60a3\u8005\u7ecf\u5e38\u4f1a\u906d\u9047\u4e25\u91cd\u7684\u590d\u53d1\u6027\u5ff5\u73e0\u83cc\u611f\u67d3\u3002APECED\u60a3\u8005\u5b58\u5728\u4e2d\u67a2\u8010\u53d7\u6027\u7f3a\u9677\uff0c\u5e76\u5177\u6709\u591a\u79cd\u7c7b\u578b\u7684\u81ea\u8eab\u514d\u75ab\u3002\u4e00\u4e9bAPECED\u60a3\u8005\u4ea7\u751f\u6297\u767d\u7ec6\u80de\u4ecb\u7d2017\uff08IL-17\uff09\u7684\u81ea\u8eab\u6297\u4f53\uff0c\u5bfc\u81f4\u5ff5\u73e0\u83cc\u53d7\u635f\u53cd\u5e94\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u5176\u4ed6\u6ca1\u6709\u9057\u4f20\u7f3a\u9677\u7684\u4e2a\u4f53\u4f1a\u4ea7\u751f\u6297\u5e72\u6270\u7d20\uff08IFN\uff09-\u03b3\u7684\u6297\u4f53\u3002\u800cIFN-\u03b3\u5bf9\u4e8e\u4e00\u4e9b\u75c5\u539f\u4f53\u7684\u63a7\u5236\u8d77\u5230\u5173\u952e\u4f5c\u7528\uff0c\u56e0\u6b64\u8fd9\u4e9b\u5b58\u5728\u6297\u5e72\u6270\u7d20\u6297\u4f53\u7684\u4e2a\u4f53\u4f1a\u53cd\u590d\u51fa\u73b0\u5206\u679d\u6746\u83cc\u611f\u67d3\u7684\u95ee\u9898\uff08\u7b2c23\u7ae0<\/span>\uff09\u3002\u7531\u4e8e\u672a\u77e5\u539f\u56e0\uff0c\u8fd9\u79cd\u60c5\u51b5\u4f3c\u4e4e\u4e3b\u8981\u53d1\u751f\u5728\u4e1c\u5357\u4e9a\u8840\u7edf\u4e2d\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

Fig.32.2 <\/span>\u4ee5T\u7ec6\u80de\u7f3a\u9677\u4e3a\u4e3b\u7684\u75be\u75c5\uff0c\u5982DiGeorge\u7efc\u5408\u5f81\u3001ZAP-70\u548cJAK\u7f3a\u9677\uff1bB\u7ec6\u80de\u75be\u75c5\uff0c\u5982X-\u8fde\u9501\u65e0\u4e19\u79cd\u7403\u86cb\u767d\u8840\u75c7<\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

\u4e25\u91cd\u8054\u5408\u514d\u75ab\u7f3a\u9677\u7684\u7b5b\u67e5<\/span><\/strong><\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u5982\u679c\u4e0d\u8fdb\u884c\u5e72\u7ec6\u80de\u79fb\u690d\u6cbb\u7597\uff0c\u51fa\u751f\u65f6\u60a3\u6709\u4e25\u91cd\u8054\u5408\u514d\u75ab\u7f3a\u9677\uff08SCID\uff09\u7684\u5a74\u513f\u5f88\u5feb\u5c31\u4f1a\u6b7b\u4ea1\u3002\u5e72\u7ec6\u80de\u7684\u79fb\u690d<\/strong>\u53ef\u4ee5\u6cbb\u6108SCID\uff0c\u4f46\u5fc5\u987b\u53ca\u65f6\u5b8c\u6210\uff0c\u82e5\u5728\u51fa\u751f\u540e\u4e0d\u4e45\u8fdb\u884c\u5e72\u9884\uff0c90%\u7684\u5a74\u513f\u53ef\u4ee5\u5b58\u6d3b\uff0c\u800c\u63a8\u8fdf\u51e0\u4e2a\u6708\uff0c\u5219\u53ea\u670950%\u7684\u5a74\u513f\u5728\u79fb\u690d\u540e\u5b58\u6d3b\u3002\u51fa\u4e8e\u8fd9\u4e9b\u539f\u56e0\uff0c\u8bb8\u591a\u56fd\u5bb6\u4f1a\u5bf9\u65b0\u751f\u513f\u8fdb\u884cSCID\u7b5b\u67e5\uff0c\u4ee5\u4fbf\u5bf9SCID\u75c5\u4f8b\u8fdb\u884c\u65e9\u671f\u8bca\u65ad\u548c\u6cbb\u7597\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

SCID\u7b5b\u67e5<\/strong>\u53ef\u5728\u5e72\u8840\u6591\uff08Guthrie\u6591\uff09\u4e0a\u8fdb\u884c\u3002\u7b5b\u9009\u8bd5\u9a8c\u4e3b\u8981\u68c0\u6d4b\u7684\u662fT\u7ec6\u80de\u53d7\u4f53\uff08TCR\uff09\u57fa\u56e0\u7247\u6bb5\u5728\u80f8\u817a\u4e2d\u53d1\u751f\u57fa\u56e0\u91cd\u6392\u65f6\u4ea7\u751f\u7684T\u7ec6\u80de\u5207\u9664\u73af\uff08TREC\uff09<\/strong>\u7684\u5b58\u5728\u3002\u5728\u91cd\u7ec4\u8fc7\u7a0b\u4e2d\u672a\u7528\u5230\u7684TCR\u57fa\u56e0\u7247\u6bb5\u4f1a\u88ab\u79fb\u9664\uff0c\u5e76\u88ab\u653e\u7f6e\u5728\u5207\u9664\u73af\u4e2d\uff08Fig.32.4<\/span>\uff09\uff0c\u5207\u9664\u73af\u4e2d\u7684DNA\u5b58\u5728\u4e8e\u4e00\u4e2a\u5b50\u7ec6\u80de\u5185\uff0c\u5728\u6709\u4e1d\u5206\u88c2\u8fc7\u7a0b\u4e2d\u4e0d\u4f1a\u590d\u5236\uff0c\u968f\u7740\u6709\u4e1d\u5206\u88c2\u7684\u8fdb\u884c\uff0cTREC\u542b\u91cf\u9010\u6e10\u88ab\u7a00\u91ca\u3002\u56e0\u6b64\uff0c\u8840\u6db2\u4e2d\u5927\u91cf\u7684TREC\u662f\u80f8\u817a\u4ea7\u751fT\u7ec6\u80de\u7684\u6807\u5fd7\uff0c\u4f4e\u6570\u91cf\u6216\u7f3a\u4e4fTREC\u5219\u8868\u660e\u672a\u80fd\u6b63\u5e38\u4ea7\u751fT\u7ec6\u80de<\/strong>\u3002TREC\u7b5b\u67e5\u53ef\u4ee5\u68c0\u6d4bSCID\u548c\u5176\u4ed6T\u7ec6\u80de\u6570\u91cf\u51cf\u5c11\u7684\u75be\u75c5\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
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Fig.32.3<\/span> \u8be5\u60a3\u8005\u5728\u7f16\u7801\u8865\u4f53C2\u548cC4\u4ee5\u53ca\u7518\u9732\u805a\u7cd6\u7ed3\u5408\u51dd\u96c6\u7d20\u7684\u57fa\u56e0\u4e2d\u9057\u4f20\u4e86\u591a\u6001\u6027\u3002\u8fd9\u4e9b\u90fd\u4f1a\u964d\u4f4e\u8865\u4f53\u7ea7\u8054\u7684\u6d3b\u6027\uff0c\u4f7f\u60a3\u8005\u5bb9\u6613\u53d7\u5230\u4e0d\u5bfb\u5e38\u7684\u611f\u67d3\u3002\u5728\u8fd9\u5f20\u8ba1\u7b97\u673a\u65ad\u5c42\u626b\u63cf\u4e2d\uff0c\u5927\u8111\u76ae\u5c42\u7684\u73af\u5f62\u5f3a\u5316\u75c5\u53d8\u662f\u5178\u578b\u7684\u8111\u8113\u80bf\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u7684\u8bca\u65ad<\/span><\/strong>
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\u56e0\u4e3a\u60a3\u6709SCID<\/strong>\u7684\u513f\u7ae5\u7684T\u7ec6\u80de\u548cB\u7ec6\u80de\u6709\u7f3a\u9677\uff0c\u4ed6\u4eec\u5728\u51fa\u751f\u540e\u7684\u51e0\u5468\u5185\u5c31\u4f1a\u53d7\u5230\u611f\u67d3\u3002\u60a3\u6709SCID\u7684\u513f\u7ae5\u901a\u5e38\u8868\u73b0\u4e3a\u5f02\u5e38\u6216\u53cd\u590d\u611f\u67d3\u3001\u53d1\u80b2\u4e0d\u826f\u3001\u8179\u6cfb\u3001\u5f02\u5e38\u76ae\u75b9\u3001\u65b0\u751f\u513f\u6b7b\u4ea1\u6216\u6709\u5bb6\u65cf\u75c5\u53f2\uff0c\u4ee5\u53ca\u6dcb\u5df4\u7ec6\u80de\u603b\u6570\u975e\u5e38\u4f4e(\u4f4e\u4e8e1\u00d7109<\/sup>\/L[106<\/sup>\/mL])\u3002\u5bf9\u4e8e\u8fd9\u7c7b\u7591\u4f3cT\u7ec6\u80de\u514d\u75ab\u7f3a\u9677\u7684\u5a74\u513f\uff0c\u53ef\u4ee5\u7528\u6d41\u5f0f\u7ec6\u80de\u4eea\u6d4b\u5b9a\u6dcb\u5df4\u7ec6\u80de\u6570\u91cf\u6765\u8fdb\u884c\u68c0\u6d4b(\u7b2c\u4e94\u7ae0<\/span>)\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u6297\u4f53\u7f3a\u4e4f\u75c7<\/strong>\u5728\u5a74\u513f\u51fa\u751f\u540e\u4f1a\u624d\u4f1a\u9010\u6e10\u51fa\u73b0\u75c7\u72b6\uff0c\u56e0\u4e3a\u5a74\u513f\u51fa\u751f\u65f6\u6bcd\u4f53\u514d\u75ab\u7403\u86cb\u767d\u4f1a\u901a\u8fc7\u80ce\u76d8\u8f6c\u79fb\uff0c\u8fd9\u53ef\u4ee5\u5728\u5a74\u513f\u51fa\u751f\u540e\u7684\u524d\u51e0\u4e2a\u6708\u4e3a\u4ed6\u4eec\u63d0\u4f9b\u4fdd\u62a4\uff0c\u67d0\u4e9b\u5f62\u5f0f\u7684\u6297\u4f53\u7f3a\u4e4f\uff0c\u5982CVID\uff0c\u751a\u81f3\u76f4\u5230\u6210\u5e74\u624d\u4f1a\u8868\u73b0\u51fa\u6765\u3002<\/p>\n

\u6297\u4f53\u7f3a\u4e4f\u6700\u5e38\u89c1\u7684\u6307\u5f81\u662f\u6162\u6027\u6216\u590d\u53d1\u6027\u7ec6\u83cc\u6027\u547c\u5438\u9053\u611f\u67d3\uff0c\u901a\u5e38\u68c0\u6d4b\u7684\u662fIgG\u3001IgA\u548cIgM\u542b\u91cf\u3002\u5bf9\u4e8e\u514d\u75ab\u7403\u86cb\u767d\u6c34\u5e73\u4f4e\u7684\u60a3\u8005\uff0c\u9996\u5148\u5e94\u6392\u9664\u7ee7\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u7684\u539f\u56e0\uff0c\u5982\u80a0\u9053\u6216\u80be\u810f\u7684\u86cb\u767d\u8d28\u4e22\u5931\u3002\u5982\u679c\u603bIgS\u6b63\u5e38\uff0c\u5219\u5e94\u68c0\u6d4b\u9488\u5bf9\u55dc\u8840\u6746\u83cc\u548c\u80ba\u708e\u7403\u83cc\u7684\u7279\u5f02\u6027\u6297\u4f53\u3002\u5982\u679c\u4e0a\u8ff0\u6d4b\u8bd5\u90fd\u6b63\u5e38\uff0c\u8fd8\u9700\u8981\u786e\u4fdd\u8865\u4f53\u6216\u795e\u7ecf\u8425\u517b\u529f\u80fd\u6ca1\u6709\u660e\u663e\u95ee\u9898\uff0c\u5982\u6162\u6027\u8089\u82bd\u80bf\u6027\u75be\u75c5\uff08CGD\uff0c\u7b2c21\u7ae0<\/span>\uff09\uff0c\u8fd9\u6837\u624d\u80fd\u5f97\u51fa\u6ca1\u6709\u514d\u75ab\u7f3a\u9677\u7684\u7ed3\u8bba\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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Fig.32.4<\/span> T\u7ec6\u80de\u53d7\u4f53(TCR)\u5207\u9664\u73af\u5728T\u7ec6\u80de\u53d1\u80b2\u8fc7\u7a0b\u4e2d\u53ea\u4ea7\u751f\u4e00\u6b21<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u6cbb \u7597<\/strong><\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u6cbb\u7597\u7684\u76ee\u7684\u662f\u9884\u9632\u611f\u67d3\u3002\u5728\u8f7b\u5ea6<\/strong>\u514d\u75ab\u7f3a\u9677\u7684\u60c5\u51b5\u4e0b\uff08\u5982\u7279\u5f02\u6027\u6297\u4f53\u7f3a\u4e4f\uff09\uff0c\u4f7f\u7528\u9884\u9632\u6027\u6297\u751f\u7d20\u53ef\u80fd\u5c31\u8db3\u591f\u4e86\uff1b\u800c\u5728\u66f4\u4e25\u91cd<\/strong>\u7684\u6297\u4f53\u7f3a\u4e4f\u75c7\u4e2d\uff0c\u9700\u8981\u514d\u75ab\u7403\u86cb\u767d\u66ff\u4ee3\u6cbb\u7597\u3002\u6ce8\u5c04\u514d\u75ab\u7403\u86cb\u767d\u662f\u4e00\u79cd\u88ab\u52a8\u514d\u75ab\u7597\u6cd5\uff08\u7b2c4\u7ae0\uff0c\u89c1Box 4.2<\/span>\uff09\uff0c\u9700\u8981\u6ce8\u5c04\u9488\u5bf9\u591a\u79cd\u75c5\u539f\u4f53\u7684\u6297\u4f53\uff0c\u56e0\u6b64\uff0c\u901a\u5e38\u4f1a\u4f7f\u7528\u6570\u5343\u540d\u6b63\u5e38\u6350\u8d60\u8005\u7684\u514d\u75ab\u7403\u86cb\u767d\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u732e\u8840\u8005\u9700\u8981\u8fdb\u884cHIV\u3001\u4e59\u809d\u3001\u4e19\u809d\u6297\u4f53\u7b5b\u67e5\u3002\u867d\u7136IgG\u7684\u63d0\u53d6\u5236\u4f5c\u8fc7\u7a0b\u4f1a\u7834\u574f\u8bb8\u591a\u75c5\u539f\u4f53\uff0c\u4f46\u901a\u5e38\u8fd8\u9700\u8981\u91c7\u53d6\u8fdb\u4e00\u6b65\u7684\u6d88\u6bd2\u63aa\u65bd\uff0c\u5982\u5df4\u6c0f\u6740\u83cc(\u52a0\u70ed\u523056\u00b0C)\u6216\u6dfb\u52a0\u6d17\u6da4\u5242\uff0c\u4ee5\u964d\u4f4e\u4f20\u64ad\u4e19\u578b\u809d\u708e\u7684\u98ce\u9669\u3002<\/p>\n

\u5982\u679c\u6000\u7591\u662fSCID\uff0c\u5e76\u4e14\u5df2\u6392\u9664HIV\u611f\u67d3\uff0c\u5e94\u5c06\u5a74\u513f\u8f6c\u8bca\u5230\u4e13\u79d1\u4e2d\u5fc3\uff0c\u5728\u90a3\u91cc\u786e\u8ba4\u8bca\u65ad\u5e76\u7ed9\u4e88\u660e\u786e\u6cbb\u7597(\u901a\u5e38\u662f\u5e72\u7ec6\u80de\u79fb\u690d[SCT])\u3002\u5728\u6b64\u4e4b\u524d\uff0c\u9700\u8981\u6ce8\u610f\u907f\u514d\u60a3\u8005\u906d\u53d7\u4e25\u91cd\u611f\u67d3\uff0c\u5982\u907f\u514d\u63a5\u79cd\u6d3b\u75ab\u82d7(\u5982\u9ebb\u75b9\u3001\u816e\u817a\u708e\u3001\u98ce\u75b9\u3001\u810a\u9ad3\u7070\u8d28\u708e)\uff0c\u4ee5\u53ca\u4f7f\u7528\u9884\u9632\u63aa\u65bd\u6765\u9884\u9632\u673a\u4f1a\u6027\u611f\u67d3\uff0c\u5982\u5409\u7f57\u7ef4\u6c0f\u80ba\u5b62\u5b50\u866b\u80ba\u708e\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u5bf9\u4e8eSCID\u548c\u5927\u591a\u6570T\u7ec6\u80de\u7f3a\u9677\u7684\u60a3\u8005\uff0c\u53ef\u80fd\u9700\u8981\u63a5\u53d7SCT\uff0c\u5728\u7b2c34\u7ae0<\/span>\u4e2d\u5c06\u5bf9SCT\u8fdb\u884c\u66f4\u5168\u9762\u7684\u4ecb\u7ecd\u3002\u5982\u679c\u5a74\u513f\u5728\u51fa\u73b0\u611f\u67d3\u4e4b\u524d\uff0c\u5373\u5728\u51fa\u751f\u540e\u51e0\u5468\u5185\u5b8c\u6210SCT\uff0c\u8fd9\u6837\u7684\u6548\u679c\u662f\u6700\u4f73\u7684\uff0c\u6210\u529f\u7387\u80fd\u8fbe\u523090%\u3002\u82e5\u56e0\u4e3a\u6ca1\u6709\u5408\u9002\u7684\u4f9b\u4f53\u7b49\u539f\u56e0\uff0c\u65e0\u6cd5\u8fdb\u884cSCT\u65f6\uff0c\u53ef\u80fd\u4f1a\u5c1d\u8bd5\u5bf9T\u7ec6\u80de\u7f3a\u9677\u7684\u60a3\u8005\u8fdb\u884c\u57fa\u56e0\u6cbb\u7597\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u57fa\u56e0\u7597\u6cd5<\/p>\n<\/section>\n

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\u57fa\u56e0\u7597\u6cd5\u4f7f\u7528\u91cd\u7ec4\u6280\u672f\u6765\u7ea0\u6b63\u60a3\u8005\u81ea\u8eab\u5e72\u7ec6\u80de\u4e2d\u7684\u9057\u4f20\u7f3a\u9677\uff0c\u7136\u540e\u8fd9\u4e9b\u5e72\u7ec6\u80de\u5c31\u53ef\u4ee5\u91cd\u5efa\u514d\u75ab\u7cfb\u7edf\u3002\u76ee\u524d\u5df2\u7ecf\u5c06\u5b83\u7528\u4e8e\u4e00\u4e9b\u6ca1\u6709\u5408\u9002\u5e72\u7ec6\u80de\u4f9b\u4f53\u7684SCID\u60a3\u8005\u3002\u76ee\u524d\u4e3a\u6b62\uff0c\u57fa\u56e0\u7597\u6cd5\u5728\u5176\u4ed6\u9057\u4f20\u6027\u75be\u75c5\u4e2d\u8fd8\u6ca1\u6709\u53d6\u5f97\u6210\u529f\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u8981\u4f7f\u57fa\u56e0\u6cbb\u7597\u53d6\u5f97\u6210\u529f\uff0c\u5fc5\u987b\u6ee1\u8db3\u51e0\u4e2a\u6761\u4ef6\uff1a<\/p>\n

1.\u5fc5\u987b\u786e\u5b9a\u6bcf\u4e2a\u60a3\u8005\u7684\u57fa\u56e0\u7a81\u53d8\uff0c\u5e76\u4e14\u5fc5\u987b\u786e\u4fdd\u7ea0\u6b63\u8fd9\u4e9b\u7a81\u53d8\u786e\u5b9e\u4f1a\u6539\u5584\u60a3\u8005\u7684\u75c5\u60c5\u3002\u4e00\u540d\u5df2\u88ab\u8bca\u65ad\u4e3aX\u8fde\u9501SCID\u7684\u513f\u7ae5(\u7b2c12\u7ae0<\/span>)\u63a5\u53d7\u4e86\u57fa\u56e0\u6cbb\u7597\uff0c\u7ea0\u6b63\u7a81\u53d8\u540e\uff0c\u4ed6\u7684\u5355\u4e2a\u6838\u6dcb\u5df4\u6837\u524d\u4f53\u7ec6\u80de\u7684\u5171\u540c\u03b3\u94fe\u7684\u7f3a\u9677\u5f97\u5230\u4fee\u590d\u3002\u8fd9\u79cd\u9006\u8f6c\u4f7f\u7ec6\u80de\u80fd\u591f\u6b63\u5e38\u589e\u6b96\uff0c\u5e76\u90e8\u5206\u5730\u6062\u590d\u4e86\u8fd9\u4e2a\u5b69\u5b50\u7684\u6dcb\u5df4\u7ec6\u80de\u6570\u91cf\uff0c\u8fd9\u8bc1\u660e\u7ea0\u6b63SCID\u4e2d\u7684\u7a81\u53d8\u662f\u5177\u6709\u53ef\u884c\u6027\u7684\u3002\u4e3a\u4e86\u8ba9\u57fa\u56e0\u7597\u6cd5\u53d1\u6325\u4f5c\u7528\uff0c\u57fa\u56e0\u5fc5\u987b\u80fd\u591f\u5b89\u5168\u5730\u5230\u8fbe\u7ec6\u80de\u5185\u3002\u4e00\u822c\u7684\u65b9\u6cd5\u662f\u4eceSCID\u60a3\u8005\u7684\u8840\u6db2\u4e2d\u56de\u6536\u5e72\u7ec6\u80de\uff0c\u5c06\u8fd9\u4e9b\u5e72\u7ec6\u80de\u7528\u6b63\u5e38\u57fa\u56e0\u8f6c\u67d3\uff0c\u901a\u5e38\u91c7\u7528\u75c5\u6bd2\u6765\u4f20\u9012\u6b63\u5e38\u57fa\u56e0(Fig.32.5<\/span>)\uff0c\u6cbb\u7597\u6240\u8f6c\u67d3\u7684\u5e72\u7ec6\u80de\u6570\u91cf\u7ea6500\u4e2a\u3002<\/p>\n

2.\u8f6c\u67d3\u7684\u57fa\u56e0\u5fc5\u987b\u5177\u6709\u589e\u6b96\u6216\u751f\u5b58\u4f18\u52bf\u3002\u5728X\u8fde\u9501\u7684SCID\u4e2d\uff0c\u7a81\u53d8\u4f4d\u4e8e\u5171\u540c\u7684\u03b3\u94fe\u4e2d\uff0c\u8be5\u94fe\u6784\u6210T\u7ec6\u80de\u751f\u957f\u56e0\u5b50(IL-2\uff0cIL-7)\u53d7\u4f53\u7684\u4e00\u90e8\u5206\uff0c\u80fd\u591f\u6b63\u5e38\u884c\u4f7f\u8be5\u53d7\u4f53\u529f\u80fd\u7684\u7ec6\u80de\u5c31\u53ef\u4ee5\u589e\u6b96\u548c\u5b58\u6d3b\u3002\u6700\u521d\u8f6c\u67d3\u7684500\u4e2a\u7ec6\u80de\uff0c\u572812\u4e2a\u5206\u88c2\u5468\u671f\u540e\u4f1a\u4ea7\u751f100\u4e07\u4e2a\u5b50\u4ee3T\u7ec6\u80de\u524d\u4f53\uff0c\u6bcf\u4e2a\u90fd\u6709\u4e0d\u540c\u7684T\u7ec6\u80de\u53d7\u4f53\uff0c\u8fd9\u4e9b\u7ec6\u80de\u8db3\u4ee5\u91cd\u5efa\u6240\u6709\u7c7b\u578b\u7684T\u7ec6\u80de\u7fa4\u3002<\/p>\n

3.\u9700\u8981\u6ce8\u610f\u7684\u662f\uff0c\u5728\u4f7f\u7528\u57fa\u56e0\u6cbb\u7597\u65f6\u4e0d\u80fd\u8bf1\u53d1\u6076\u6027\u80bf\u7624\u3002\u5982\u679c\u5728\u764c\u57fa\u56e0\u65c1\u8fb9\u63d2\u5165\u4e00\u4e2a\u5177\u6709\u6d3b\u6027\u7684\u542f\u52a8\u5b50\u57fa\u56e0\uff0c\u5219\u764c\u57fa\u56e0\u53ef\u80fd\u88ab\u6fc0\u6d3b\u5e76\u5bfc\u81f4\u764c\u75c7\u3002\u5728\u6700\u521d\u63a5\u53d7X\u8fde\u9501SCID\u57fa\u56e0\u6cbb\u7597\u768420\u540d\u60a3\u8005\u4e2d\uff0c\u67095\u540d\u60a3\u8005\u56e0\u666e\u901a\u03b3\u94fe\u589e\u5f3a\u5b50\u63d2\u5165\u4e86\u764c\u57fa\u56e0LMO2\u800c\u53d1\u5c55\u4e3a\u6dcb\u5df4\u6076\u6027\u80bf\u7624\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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\u5728\u5176\u4ed6\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u4e2d\u4e5f\u5c1d\u8bd5\u7740\u4f7f\u7528\u4e86\u57fa\u56e0\u7597\u6cd5\uff0c\u5982CGD(\u7b2c21\u7ae0<\/span>)\uff0c\u8fd9\u79cd\u75be\u75c5\u53ef\u4ee5\u4ea7\u751f\u6b63\u5e38\u6570\u91cf\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\uff0c\u4f46\u4e2d\u6027\u7c92\u7ec6\u80de\u672a\u7ecf\u6c27\u5316\u5e94\u6fc0\uff0c\u800c\u6c27\u5316\u5e94\u6fc0\u901a\u5e38\u662f\u7528\u4e8e\u6740\u6b7b\u75c5\u539f\u4f53\u7684\u3002\u5728\u8be5\u75be\u75c5\u7684\u6cbb\u7597\u4e2d\uff0c\u67d0\u4e9b\u5e72\u7ec6\u80de\u4e2d\u7684\u7a81\u53d8\u5f97\u5230\u7ea0\u6b63\u540e\u4ea7\u751f\u4e86\u4e00\u4e9b\u6b63\u5e38\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\uff0c\u4f46\u672a\u80fd\u53d6\u5f97\u957f\u671f\u7684\u6210\u529f\u3002<\/p>\n

\u5728\u6700\u521d\u7684\u51e0\u5468\uff0c\u51e0\u4e4e\u6ca1\u6709\u4ea7\u751f\u6b63\u5e38\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\u3002\u8fd9\u53ef\u80fd\u662f\u56e0\u4e3a\u7ecf\u8fc7\u4fee\u6b63\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\u524d\u4f53\u4e0e\u672a\u4fee\u6b63\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\u76f8\u6bd4\u5e76\u6ca1\u6709\u589e\u6b96\u6216\u5b58\u6d3b\u4f18\u52bf\uff0c\u5bfc\u81f4\u643a\u5e26\u6b63\u5e38\u57fa\u56e0\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\u4e0d\u80fd\u5b8c\u5168\u8fdb\u5165\u9aa8\u9ad3\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
\n
\n

\u56ca\u6027\u7ea4\u7ef4\u5316\u662f\u6700\u5e38\u89c1\u7684\u9057\u4f20\u75c5\u3002\u7a81\u53d8\u53d1\u751f\u5728CFTR\u57fa\u56e0\u4e2d\uff0c\u8be5\u57fa\u56e0\u901a\u5e38\u5728\u547c\u5438\u9053\u3001\u80a0\u9053\u3001\u809d\u810f\u548c\u80f0\u817a\u7684\u4e0a\u76ae\u7ec6\u80de\u4e2d\u8868\u8fbe\u3002\u867d\u7136\u8fd9\u79cd\u5f02\u5e38\u57fa\u56e0\u5df2\u7ecf\u88ab\u53d1\u73b0\u591a\u5e74\uff0c\u5e76\u4e14\u5df2\u7ecf\u751f\u4ea7\u51fa\u4e86\u5e26\u6709\u6b63\u786e\u57fa\u56e0\u7684\u9006\u8f6c\u5f55\u75c5\u6bd2\u8f7d\u4f53\uff0c\u4f46\u5b9e\u9a8c\u8bc1\u660e\uff0c\u8981\u8f6c\u67d3\u8fd9\u4e9b\u9776\u7ec6\u80de\u662f\u975e\u5e38\u56f0\u96be\u7684\u3002<\/p>\n

\u4e00\u90e8\u5206\u7684\u539f\u56e0\u662f\uff0c\u4e0e\u9020\u8840\u5e72\u7ec6\u80de\u4e0d\u540c\uff0c\u4ece\u547c\u5438\u9053\u83b7\u53d6\u7ec6\u80de\u6765\u8fdb\u884c\u57fa\u56e0\u8f6c\u67d3\u672c\u8eab\u5c31\u4e0d\u5bb9\u6613\u3002\u6b64\u5916\uff0c\u5982\u679c\u6b63\u5e38\u57fa\u56e0\u88ab\u6210\u529f\u5730\u8f6c\u67d3\u548c\u8868\u8fbe\uff0c\u5c06\u4f1a\u4ea7\u751f\u53e6\u4e00\u4e2a\u91cd\u5927\u7684\u7406\u8bba\u95ee\u9898\uff1a\u514d\u75ab\u7cfb\u7edf\u4f1a\u5c06CFTR\u57fa\u56e0\u4ea7\u7269\u8bc6\u522b\u4e3a\u201c\u5916\u6765\u201d\u86cb\u767d\u5e76\u4ea7\u751f\u514d\u75ab\u53cd\u5e94\u5417\uff1f\u7531\u4e8e\u4e0a\u8ff0\u539f\u56e0\uff0c\u57fa\u56e0\u6cbb\u7597\u5728SCID\u4e2d\u5c1a\u65e0\u826f\u597d\u7684\u5e94\u7528\u524d\u666f\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

Fig.32.5<\/span> X\u8fde\u9501\u4e25\u91cd\u8054\u5408\u514d\u75ab\u7f3a\u9677\u7684\u57fa\u56e0\u6cbb\u7597\u6210\u529f\u6709\u4e24\u4e2a\u539f\u56e0\uff1a\u7b2c\u4e00\uff0c\u83b7\u53d6\u5e72\u7ec6\u80de\u76f8\u5bf9\u7b80\u5355\uff1b\u7b2c\u4e8c\uff0c\u8f6c\u67d3\u540c\u6837\u03b3\u94fe\u7684\u7ec6\u80de\u80fd\u591f\u589e\u6b96<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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BOX 32.1 <\/span>\u5ef6\u8bef\u7684\u8bca\u65ad<\/p>\n<\/section>\n<\/section>\n

\n
<\/svg><\/section>\n
<\/svg><\/section>\n<\/section>\n
\n
\n

25\u5c81\u60a3\u8005\uff0c\u5973\u6027\uff08\u5927\u5b66\u751f\uff09\uff0c\u6392\u75f0\u52a0\u91cd\uff0c\u547c\u5438\u6025\u4fc3\uff0c\u80f8\u90e8\u611f\u67d3\u53cd\u590d\u53d1\u4f5c\uff0c\u56e0\u800c\u65e0\u6cd5\u5b8c\u6210\u5b66\u4e1a\u3002\u8fc7\u53bb\u76843\u5e74\u91cc\u5979\u75c7\u72b6\u6301\u7eed\u4e0d\u65ad\uff1b\u5e76\u60a3\u6709\u6162\u6027\u9f3b\u7aa6\u708e\uff0c\u505a\u8fc7\u4e09\u6b21\u9f3b\u7aa6\u5f15\u6d41\u624b\u672f(\u5747\u672a\u6210\u529f)\uff0c\u4e0d\u5438\u70df\uff0c\u4e14\u5bb6\u65cf\u53f2\u65e0\u5f02\u5e38\u3002<\/p>\n

CT\u7ed3\u679c\u663e\u793a(Fig.32.6<\/span>)\u80ba\u90e8\u6709\u652f\u6c14\u7ba1\u6269\u5f20\uff0c\u652f\u6c14\u7ba1\u6269\u5f20\u662f\u547c\u5438\u9053\u4e2d\u4e0d\u53ef\u9006\u8f6c\u7684\u635f\u4f24\uff0c\u591a\u7531\u4e8e\u53cd\u590d\u53d1\u4f5c\u7684\u611f\u67d3\u9020\u6210\u7684\uff0c\u75f0\u4e2d\u68c0\u6d4b\u5230\u55dc\u8840\u6746\u83cc\uff0c\u514d\u75ab\u5b66\u68c0\u67e5\u7ed3\u679c\u5982Table 32.2<\/span>\u6240\u793a\u3002<\/p>\n

\u6392\u9664\u7ee7\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u7684\u539f\u56e0\u540e\uff0c\u5979\u63a5\u79cd\u542b\u6709\u80ba\u708e\u7403\u83cc\u591a\u7cd6\u7684\u75ab\u82d7\u3002\u8fdb\u4e00\u6b65\u7684\u68c0\u6d4b\u663e\u793a\uff0c\u5979\u5bf9\u75ab\u82d7\u6ca1\u6709\u4ea7\u751f\u53cd\u5e94\uff0c\u7b26\u5408\u5e38\u89c1\u53d8\u5f02\u578b\u514d\u75ab\u7f3a\u9677\u75c5(CVID)\u7684\u8bca\u65ad\u6807\u51c6\u3002\u5979\u7684\u75c7\u72b6\u5728\u9759\u8109\u6ce8\u5c04\u514d\u75ab\u7403\u86cb\u767d\u540e\u6709\u6240\u6539\u5584\uff0c\u987a\u5229\u6bd5\u4e1a\u3002<\/p>\n

CVID\u901a\u5e38\u53d1\u751f\u5728\u4e8c\u5341\u5c81\u5de6\u53f3\uff0c\u60a3\u8005\u5728\u63a5\u53d7\u4e86\u514d\u75ab\u7403\u86cb\u767d\u6cbb\u7597\u540e\uff0c\u547c\u5438\u9053\u611f\u67d3\u7684\u9891\u7387\u548c\u4e25\u91cd\u7a0b\u5ea6\u5c31\u4f1a\u964d\u4f4e\u3002\u4f46\u5f53\u8bca\u65ad\u5ef6\u8bef\u65f6\uff0c\u53ef\u80fd\u5df2\u7ecf\u51fa\u73b0\u4e86\u4e0d\u53ef\u9006\u8f6c\u7684\u5e76\u53d1\u75c7\u3002\u901a\u8fc7\u68c0\u67e5\u53cd\u590d\u53d1\u4f5c\u6216\u7f55\u89c1\u611f\u67d3\u60a3\u8005\u7684\u514d\u75ab\u7403\u86cb\u767d\u6c34\u5e73\u53ef\u4ee5\u9632\u6b62\u8fd9\u79cd\u5ef6\u8bef\u8bca\u65ad\u7684\u53d1\u751f\u3002<\/p>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

Table 32.2 <\/span>\u4e00\u4f8b\u5e38\u89c1\u53d8\u5f02\u578b\u514d\u75ab\u7f3a\u9677\u60a3\u8005\u7684\u514d\u75ab\u5b66\u68c0\u67e5<\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n

\n
<\/section>\n<\/section>\n
\n
\n
\n


<\/p>\n<\/section>\n<\/section>\n

\n
\n

Fig.32.6 <\/span>\u8fd9\u5f20\u80ba\u626b\u63cf\u7ed3\u679c\u663e\u793a\u4e00\u4f4d\u60a3\u6709\u5e38\u89c1\u53d8\u5f02\u578b\u514d\u75ab\u7f3a\u9677\u7684\u5987\u5973\u51fa\u73b0\u652f\u6c14\u7ba1\u6269\u5f20\u548c\u7247\u72b6\u80ba\u708e\u3002\u5728\u53f3\u80ba\uff0c\u7531\u4e8e\u5468\u56f4\u80ba\u7684\u5b9e\u53d8\uff0c\u53ef\u89c1\u652f\u6c14\u7ba1\u3002\u652f\u6c14\u7ba1\u6709\u4e9b\u6269\u5f20\uff0c\u7ba1\u8154\u4e0d\u5149\u6ed1\u3002<\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n

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\n
\n
\n
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BOX 32.2<\/span> Wiskott-Aldrich\u7efc\u5408\u5f81<\/p>\n<\/section>\n<\/section>\n

\n
<\/svg><\/section>\n
<\/svg><\/section>\n<\/section>\n
\n
\n

Fig.32.7<\/span>\u548cFig.32.8<\/span>\u4e2d\u7684\u75c5\u4eba\u6709\u7600\u4f24\u548c\u51fa\u8840\uff0c\u7ecf\u8bca\u65ad\u662f\u7531\u4e8e\u5faa\u73af\u4e2d\u7684\u8840\u5c0f\u677f\u6570\u91cf\u51cf\u5c11\u6240\u81f4\u3002\u60a3\u8005\u8fd8\u6709\u4e25\u91cd\u7684\u6e7f\u75b9\u548c\u7f55\u89c1\u7684\u75c5\u6bd2\u611f\u67d3\uff0c\u5728Fig.32.7<\/span>\u4e2d\uff0c\u9762\u90e8\u5b58\u5728\u591a\u5904\u75a3\u72b6\u75c5\u53d8\uff0c\u662f\u7531\u4e8e\u75d8\u75c5\u6bd2\u5bb6\u65cf\u6240\u5f15\u8d77\u7684\u4f20\u67d3\u6027\u8f6f\u75a3\u3002\u5728\u514d\u75ab\u7cfb\u7edf\u6b63\u5e38\u7684\u513f\u7ae5\u4e2d\uff0c\u4f20\u67d3\u6027\u8f6f\u75a3\u5f15\u8d77\u4e00\u8fc7\u6027\u611f\u67d3\u901a\u5e38\u53ea\u67091-2\u4e2a\u75c5\u7076\uff0c\u51e0\u5468\u5185\u5c31\u4f1a\u88ab\u7ec6\u80de\u6bd2\u6027T\u6dcb\u5df4\u7ec6\u80de\u6e05\u9664\u3002\u800c\u5728\u8fd9\u4f4d\u60a3\u8005\u8eab\u4e0a\u770b\u5230\u7684\u5f02\u5e38\u5e7f\u6cdb\u548c\u6301\u7eed\u7684\u611f\u67d3\u5219\u662f\u5178\u578b\u7684\u673a\u4f1a\u6027\u611f\u67d3\u3002<\/p>\n

\u540c\u65f6\uff0c\u60a3\u8005\u8fd8\u60a3\u6709B\u7ec6\u80de\u6dcb\u5df4\u7624\u7684\u6076\u6027\u80bf\u7624\u3002\u5982\u7b2c35\u7ae0<\/span>\u6240\u8ba8\u8bba\u7684\uff0c\u514d\u75ab\u7f3a\u9677\u60a3\u8005\u7684\u6dcb\u5df4\u7624\u901a\u5e38\u662f\u7531EBV\u5f15\u8d77\u7684\u3002\u4e34\u5e8a\u7279\u5f81\uff08\u8840\u5c0f\u677f\u8ba1\u6570\u4f4e\u3001\u6e7f\u75b9\u3001\u611f\u67d3\u548c\u6076\u6027\u80bf\u7624\uff09\u4f7f\u4ed6\u7684\u4e3b\u6cbb\u533b\u751f\u63a8\u65ad\u5176\u4e3aWiskott-Aldrich\u7efc\u5408\u5f81\uff0c\u8fd9\u662f\u4e00\u79cd\u539f\u53d1\u6027\u514d\u75ab\u7f3a\u9677\u75c5\u3002\u5728\u60a3\u8005\u8eab\u4e0a\u63d0\u53d6DNA\u6837\u672c\uff0c\u5e76\u5bf9Wiskott-Aldrich\u7efc\u5408\u5f81\u86cb\u767d(WASp)\u57fa\u56e0\u8fdb\u884c\u4e86\u6d4b\u5e8f\u3002\u7ed3\u679c\u8868\u660e\u60a3\u8005\u7684Wiskott-Aldrich\u7efc\u5408\u5f81\u57fa\u56e0(WAS)\u53d1\u751f\u4e86\u7a81\u53d8\uff0c\u8bc1\u5b9e\u4e86\u533b\u751f\u7684\u8bca\u65ad\u3002<\/span><\/p>\n

Wiskott-Aldrich\u7efc\u5408\u5f81\u662f\u4e00\u79cdX\u8fde\u9501\u75be\u75c5\uff0c\u4f46\u60a3\u8005\u7684\u5bb6\u65cf\u4e2d\u5e76\u6ca1\u6709\u53d7\u5f71\u54cd\u7684\u7537\u6027\u4eb2\u5c5e\u75c5\u53f2\uff0c\u8be5\u60a3\u8005\u7684\u7a81\u53d8\u4e3a\u5bb6\u65cf\u5185\u9996\u4f8b\u3002<\/p>\n

\u7ecf\u8fc7\u6cbb\u7597\u60a3\u8005\u7684\u6dcb\u5df4\u7624\u5f97\u5230\u4e86\u63a7\u5236\uff0c\u5e76\u63a5\u53d7\u4e86\u5e72\u7ec6\u80de\u79fb\u690d(\u7b2c34\u7ae0<\/span>)\u3002\u4e24\u5e74\u540e\uff0c\u4ed6\u8fc7\u4e0a\u4e86\u6b63\u5e38\u7684\u751f\u6d3b(Fig.32.8<\/span>)\u3002<\/p>\n

WASp\u8d1f\u8d23\u8c03\u8282\u808c\u52a8\u86cb\u767d\u7ec6\u80de\u9aa8\u67b6\u3002WASp\u7684\u7f3a\u9677\u963b\u788d\u4e86\u8840\u5c0f\u677f\u7684\u6b63\u5e38\u53d1\u80b2\uff08\u539f\u56e0\u672a\u6e05\uff09\u3002WASp\u7f3a\u9677\u5bf9\u514d\u75ab\u7cfb\u7edf\u7684\u5f71\u54cd\u66f4\u4e3a\u76f4\u89c2\uff0c\u6b63\u5e38T\u7ec6\u80de\u6fc0\u6d3b\u540e\uff0cZAP-70\uff08\u7b2c11\u7ae0<\/span>\uff09\u4f1a\u6fc0\u6d3bWASp\u5206\u5b50\uff0c\u4ece\u800c\u5bfc\u81f4\u7ec6\u80de\u9aa8\u67b6\u53d1\u751f\u53d8\u5316\uff0c\u5f62\u6210\u514d\u75ab\u7a81\u89e6\uff08\u7b2c16\u7ae0<\/span>\uff09\uff0c\u5e76\u5728\u7ec6\u80de\u6bd2\u6027T\u6dcb\u5df4\u7ec6\u80de\u4e2d\u5411\u9776\u7ec6\u80de\u8f93\u9001\u7a7f\u5b54\u7d20\u548c\u9897\u7c92\u9176\uff08Fig.32.9<\/span>\uff09\u3002\u800c\u5728Wiskott-Aldrich\u7efc\u5408\u5f81\u60a3\u8005\u4e2d\uff0c\u7531\u4e8e\u8fd9\u4e9bT\u7ec6\u80de\u529f\u80fd\u7684\u7f3a\u9677\uff0c\u4ed6\u4eec\u7684\u7ec6\u80de\u5185\u75c5\u6bd2\u75c5\u539f\u4f53\u65e0\u6cd5\u6e05\u9664\u3002\u6b64\u5916\uff0c\u8fd9\u4e9b\u60a3\u8005\u7684B\u7ec6\u80de\u548c\u6811\u7a81\u72b6\u7ec6\u80de\u529f\u80fd\u4e5f\u5b58\u5728\u7f3a\u9677\uff0c\u5bfc\u81f4\u6297\u4f53\u4ea7\u751f\u4e5f\u53d7\u5230\u4e86\u5f71\u54cd\u3002<\/p>\n<\/section>\n<\/section>\n

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<\/section>\n<\/section>\n
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\n


<\/p>\n<\/section>\n<\/section>\n

\n
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Fig.32.7<\/span> Wiskott-Aldrich\u7efc\u5408\u5f81\u60a3\u8005<\/span>\u8138\u4e0a\u7684\u4f20\u67d3\u6027\u8f6f\u75a3\u3002<\/span><\/p>\n<\/section>\n<\/section>\n<\/section>\n

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<\/section>\n<\/section>\n
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<\/p>\n<\/section>\n<\/section>\n

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Fig.32.8 <\/span>Fig.32.7<\/span>\u4e2d\u7ecf\u8fc7\u5e72\u7ec6\u80de\u79fb\u690d2\u5e74\u540e\u7684\u60a3\u8005\u3002<\/p>\n<\/section>\n<\/section>\n<\/section>\n

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<\/section>\n<\/section>\n
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<\/p>\n<\/section>\n<\/section>\n

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Fig.32.9 <\/span>Wiskott-Aldrich syndrome protein (WASp)\u5728T\u7ec6\u80de\u6d3b\u5316\u540e\u5bf9\u808c\u52a8\u86cb\u767d\u7ec6\u80de\u9aa8\u67b6\u7684\u7ec4\u7ec7\u8d77\u4f5c\u7528\u3002  <\/p>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n<\/section>\n

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\u6765\u6e90 | Immunology for Medical Students<\/span><\/section>\n
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